Hassan Mottaghi Moghaddam Shahri

Background: Inadequate pulmonary blood flow in the tetralogy of Fallot (ToF) can lead to the development of major aortopulmonary collaterals, interfering with surgical repair. Objectives: We aimed to depict the crucial role of cardiac catheterization before the surgical intervention of pediatrics undergoing ToF total correction. Methods: This retrospective cohort study was conducted on consecutive pediatric patients under two years of age who underwent ToF total correction surgery. All of the patients were diagnosed with ToF with doubled times trans-thoracic echocardiography, and the patients with high suspicion of the presence of major aortopulmonary collateral arteries (MAPCAs) underwent cardiac catheterization before ToF total correction surgery. Conversely, if the catheterization approach failed, they underwent ToF total correction surgery simultaneously with MAPCA closure. On the other hand, the patients with no evidence of MAPCA in the echocardiographic findings (as considered undiagnosed patients) underwent ToF total correction surgery. Results: Two hundred fifty eligible pediatrics who underwent ToF total correction surgery participated. The ICU stay (P < 0.001), hospital stay (P < 0.001), and weaning time from mechanical ventilation (P < 0.001) in patients with correct diagnosis before the ToF surgery were statistically lower than misdiagnosed open MAPCA patients who underwent the surgical approach of the ToF group. Some intra-operative and postoperative parameters showed no statistical differences between patients in the two groups. We had three mortality in the patients whose trans-thoracic echocardiography (TTE) findings were undiagnosed as there was no evidence to rely on the presence of open MAPCA. Conclusions: Based on this study's findings, we strongly recommend that all ToF patients undergo cardiopulmonary catheterization before the surgical approach.

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Patients with repaired TOF (rTOF) expose to moderate to severe pulmonary regurgitation (PR), right ventricular (RV) dysfunction, and RV failure. Although cardiac magnetic resonance (CMR) provides details of RV function and structure, an electrocardiogram (ECG) is accessible and inexpensive. Objectives: We aimed to assess the correlation between clinical symptoms and ECG parameters with CMR findings. Methods: This cross-sectional study assessed the patients with rTOF who underwent CMR between May 2020 and September 2022 in Imam Reza Hospital. The signs and symptoms of rTOF were assessed, and a pediatric cardiologist interpreted ECG parameters. We considered CMR findings, clinical symptoms, and ECG parameters with CMR findings in rTOF. In addition, fragmented QRS (fQRS), the additional spikes within the QRS complex, was used to indicate conduction disturbance due to myocardial scar or fibrosis. Data were analyzed by SPSS version 18. Results: Among 42 patients with rTOF, 20 patients were male. Although the QT correction (QTc) had a significant correlation with CMR parameters, fQRS showed a positive correlation with right ventricular ejection fraction (RVEF) less than 47% (OR 2.06, 95% CI 1.45 - 2.93; P-value = 0.005), right ventricular end-diastolic volume (RVEDV) more than 150 mL/m2 (OR 4.4, 95% CI 2.03 - 9.5; P-value = 0), and right ventricular end-systolic volume (RVESV) more than 80 mL/m2 (OR 2.83, 95% CI 1.59 - 5.04; P-value = 0). We found a murmur in the clinical examination of 64.3% of patients. Our results showed that tachycardia and tachypnea were the most common signs in these patients. Surgery at a young age was significantly correlated to lower ascending aortic volume, right pulmonary artery size, left pulmonary artery size, right atrium and left atrium volume, and main pulmonary artery flow. Conclusions: Compared to other ECG parameters, fQRS could strongly predict RVESV, RVEDV, and RVEF as the right heart indexes. Besides, repair surgery at a younger age reduces the possibility of needing pulmonary valve replacement surgery at older ages. Therefore, clinicians should consider these issues in patients.

Purpose: Sydenham chorea is known for its rapid, irregular, and aimless involuntary movements and is considered a benign and self-limiting condition among the major manifestations of rheumatic fever. The current study reviewed the demographic, clinical, and paraclinical findings of pediatric patients with Sydenham chorea.Methods: This cross-sectional study was conducted among 22 patients with Sydenham chorea who were admitted to the pediatric wards of Mashhad Imam Reza and Ghaem Hospitals between 2006 and 2016. Data from these patients’ medical records were extracted, organized using checklist forms, and analyzed.Results: Eight patients were male and 14 were female. The average age was 10.09±3.53 years. In 31.8% of patients, chorea was the only sign of rheumatic fever. Chorea was unilateral in 21.1% of patients. The most common clinical findings were, in descending order, jerky movements, facial grimacing, gait disorders, mental disorders, speech disorders, muscle weakness, and milkmaid’s grip. Cardiac auscultation was normal in 76.2% of patients, while a holosystolic murmur was heard in 23.8%. In laboratory exams, 50% of patients were erythrocyte sedimentation rate (ESR)-positive, 31.2% were C-reactive protein (CRP)-positive, and 53.3% were anti-streptolysin O (ASO)-positive. Echocardiography showed the prevalence of mitral regurgitation (63.6%), aortic regurgitation (45.5%), tricuspid regurgitation (22.7%), pulmonary regurgitation (4.5%), and pericardial effusion (4.5%).Conclusion: This study showed that Sydenham chorea can be the only sign of rheumatic fever. This disease typically occurs in children between the ages of 7 and 12. ESR, CRP, and ASO can be the most effective laboratory tests for diagnosis.

Background: To evaluate the ability in initial prenatal diagnosis of congenital heart disease (CHD), the most common considerable and also fatal congenital disorder. It can be achieved by ultrasound (US) or echocardiography (FE) which are performed by radiologists and pediatric cardiologists, respectively. Objectives: The study aimed to compare the results, confirm the modality that promotes the effectiveness of final diagnosis and optimize its management. Methods: A retrospective cross-sectional study on referred pregnant women was conducted in Imam Reza hospital, Mashhad, Iran from 2012 to 2019. Fetal echocardiography was performed by one skilled professional which was compared to ultrasound anomaly scan reports of the same fetuses. Data were analyzed using statistical package for the social sciences (SPSS) ver. 18. The categorical variables were demonstrated as percentages and the agreement between two methods was evaluated by Cohen’s kappa coefficient (K > 0.4, P < 0.05 as significant cut-off). The sensitivity and specialty, as well as the accuracy, positive and negative predictive values, were all calculated. Results: Out of 312 examinations of fetuses, 56 cases were Major cardiac diseases and 62 were Minor cases. FE discovered that 14 out of 33 normal US cases were abnormal. In addition, the US considered 52 cases as structural cardiac abnormalities, of which only 34 cases were verified by FE. The most prevalent cardiac anomaly discovered by FE was the intra-cardiac echogenic focus (12.17%), compared to 7.05% of US diagnoses. The methods considered VSD the most common major, septal CHD with different quantities. The US overdiagnosed 71.4% of cases, whereas underdiagnosed 87.5% of VSDs. The US was unable to detect 11 out of 13 complex-CHDs (84.61%). Except for one percent, the US did not report AVSD. Arrhythmia was detected in 37 cases, with the US correctly diagnosing six of them. The US underdiagnosed 35 major (mostly septal defects 45%) and 26 minor structural cases; additionally, the US overdiagnosed 10 minor and 7 major cases. The methods agreed with the content of 11.53% of the CHD diagnosis. The sensitivity, specificity, and accuracy of the US method in comparison to FE for CHD diagnosis were calculated 62%, 74.2%, and 68.7% respectively. The positive and negative predictive values were 66.1% and 70.27%, respectively. Conclusions: There was a significant correlation between the results of US and FE, but there was no significant agreement in definite diagnosis, particularly for major CHDs. Although the FE is still the best modality; the US by expert sonographers is so contributory and a screening method. It is recommended to do FE at least once during pregnancy, but it is not currently accepted according to the updated guidelines.

Background: Children who have undergone cardiac surgeries due to congenital heart disease are prone to various kinds of infections. Objectives: This study was done to investigate the prevalence of nosocomial infections and microbiology of post-cardiac surgery infections in pediatric patients with congenital heart disease (CHD). Methods: In this cross-sectional study, the epidemiology and microbiology of post-cardiac surgery for pediatric patients with CHD at Imam Reza Hospital of Mashhad University of Medical Sciences between 2014 and 2017 were investigated. Demographic and clinical information was recorded, and the findings were analyzed using SPSS 16. Results: Out of 1128 patients with open heart surgery during the four years of the study, 135 patients, including 80 males (60.1%) and 55 females (39.9%) with a mean age of 8.06 ± 3.86 months, were enrolled in the study. The prevalence of infection was 11.96%. The most common isolated bacteria were Acinetobacter (19/135, 14.1%), Pseudomonas spp. (13/135, 9.6%), and Enterobacter (13/135, 9.6%) as Gram-negative ones and Corynebacterium diphtheria (10/135, 7.4%) and Staphylococcus epidermidis (10/135, 7.4%) as Gram-positive types. Candida albicans (14/135, 10.4%) were also the most frequent fungi. The frequency of infection-causing masses did not differ significantly between different cardiac abnormalities (P = 0.831), sex (P = 0.621), age (P = 0.571), and weight (P = 0.786) groups. Also, the duration of hospitalization, intubation, bypass time, and urinary catheterization in positive culture cases were significantly longer than in negative cases. Conclusions: In our study, the most common infections in children who underwent heart surgery were Acinetobacter, C. albicans, Pseudomonas, and Enterobacter. It is suggested to reduce the hospitalization, intubation, bypass, and urinary catheterization time to reduce nosocomial infections in these patients and decrease treatment costs.

Accumulation of excess iron in heart can lead to cardiac dysfunction, which is the most common cause of death in thalassemia major patients. Biopsy is an invasive procedure and therefore not an ideal option to assess iron load. However, standard/usual non-invasive methods, such as ferritin measurement, have some limitations and the results show poor correlations with iron load. Magnetic Response Imaging (MRI-T2*), as a non-invasive and reliable method for iron load assessment in organs such as liver and heart, can be suggested as a favorable alternative. This cross-sectional study was implemented in Thalassemia and Hemophilia Clinic Center (Sarvar) affiliated with Mashhad University of Medical Sciences, Mashhad, Iran, from 2012 to 2013. After the approval of the research protocol by the local ethic committee, laboratory tests, including CBC and serum ferritin, were carried out, and echocardiography and heart and liver MRI-T2* were performed. All statistical analysis was done through SPSS software (version 11.5), using independent sample t test and Pearson’s correlation coefficient test. A P value ≤0.05 was considered to be significant. 88 patients with the mean (±SD) age of 21.2 (±5.6) years, (range 11–37 years) were observed. Iron load was assessed using MRI-T2* with the following results: Out of 88 patients, 48.9 % had mild to severe cardiac siderosis, and 75.2 % had mild to severe liver siderosis. We demonstrated a correlation between liver MRI-T2* and serum ferritin, and heart MRI-T2* and ejection fraction. However, no correlation between liver and heart MRI-T2* was observed. Heart and liver siderosis is a common and serious problem in thalassemia major patients, and MRI-T2* as a sensitive and non-invasive technique can be used for early/timely detection of siderosis and good therapeutic monitoring in these patients.

Background: Down syndrome (DS) is the most common chromosomal abnormality in newborns and is associated with other congenital malformations and health problems. The features of Down syndrome differ according to ethnicity and geographic region. Objectives: The main aim was to assess the clinical characteristics of DS patients in a referral pediatric cardiology department. Patients and Methods: In this cross-sectional study, we assessed the clinical characteristics of children with Down syndrome and heart defects in an educational hospital over 11 years (from September 2001 to September 2012) in Iran. All data were collected according to a checklist created by the researchers, which included the clinical information, genetic characteristics, cardiac and non-cardiac co-existing diseases, and parental variables of the children. An independent t-test and a chi-square test were used to compare qualitative variables such as birth weight and age of diagnosis. P < 0.05 was considered statistically significant. Results: 100 patients with Down syndrome and congenital heart disease were evaluated; 52 were female (52%) and 48 were male (48%). The average birth weight of the subjects was 2745 ± 523 (mean ± SD) grams. The mean age of the patients’ mothers was 32 ± 6 years, and the mean age of the patients’ fathers was 36 ± 6 years. Chromosomal analysis was performed for 61 patients, 60 of whom had free trisomy (98.4%), one of whom had translocation (1.6%), and none of whom had a mosaic pattern of chromosomal abnormality. The parents of 33 the patients in this study were consanguineous. All patients had cardiac disorders, but non-cardiac disorder also was recorded in 37 patients (37%). The most common non-cardiac disorder in patients was hypothyroidism, and the second most common was gastrointestinal problems. Conclusions: Parents were blood relatives in 33 (33%) of the patient cases, which is a very high rate. Therefore, non-random mating is an important issue in developing countries that needs more attention Keywords: Clinical Characteristics; Down Syndrome; Congenital Heart Disease; Developing Country